Hereditary Lymphedema and Its Types
By Peter Hodges
Hereditary lymphedema, also known as primary lymphedema is genetically inherited and is caused due to a deformed lymphatic system at birth. A condition where the lymphatic fluid accumulated in one area causes swelling and pain is described as lymphedema. Hereditary lymphedema may take three different forms which are discussed below:
1. Milroy’s Disease: This is the first type of hereditary lymphedema that can be present at the birth of the individual as a result of inheriting a particular gene. The condition is mostly present in one or, at times, in both legs. Family history along with the presence of a swelling in the legs confirm the diagnosis. Further tests are conducted by injecting a dye in the affected leg. This dye is traced by a computer to detect the blocks in the lymph vessels. Although there is no known cure for the ailment, it can be controlled with the aid of decongestive therapy. Lymphedema patients may encounter complications like cellulitis, lymphangitis or fibrotic limb tissues if treatment is not begun at the early stages.
2. Lymphedema Praecox Meige, also called Hereditary Lymphedema Type II is the most common of all hereditary lymphedema conditions. The disease shows up at the time of puberty and is evident in the legs. Symptoms like a swelling below the waist area, redness, irritation and discomfort. Usually family history shows a similar kind of swelling that confirms the diagnosis. Yellow nail syndrome and pulmonary hypertension are some other indications. Decongestive therapy right from the initial stage is suggested as a method of treatment for Lymphedema Praecox Meige.
3. Lymphedema Tarda/ Delayed Onset Lymphedema or Hereditary Lymphedema Type III: This lymphedema condition shows up after 35 years of age and involves swelling of the legs. The women are more liable to get this disease than men. Like in other hereditary lymphedema conditions, here too a family history of the disease is seen. Correct diagnosis with the help of certain tests is essential to initiate the right treatment. Beginning a decongestive therapy in the early stages might help to manage the condition better.
Malformation of the lymphatic system right from the time of the birth of the individual is the cause of hereditary lymphedema. The patient may possess very few lymph glands or he may have an excess of lymph glands. Either way, there is a problem because the lymphatic system is not properly developed. Since the condition is genetic, it cannot be prevented nor cured, but with due care, the symptoms can be controlled. Skin hygiene is very important. The feet must be given extra attention so as to avoid infection in the legs. Skin creams and ointments should be used to keep the skin soft and hydrated. Dry skin may cause cracks, an invitation to infection, which must be avoided at all costs. Fungal infections must not be ignored and should be treated to prevent further complications.
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